-
1
-
-
0037444446
-
Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein
-
Adachi H., Katsuno M., Minamiyama M. et al. (2003) Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein. J. Neurosci. 23, 2203-2211.
-
(2003)
J. Neurosci.
, vol.23
, pp. 2203-2211
-
-
Adachi, H.1
Katsuno, M.2
Minamiyama, M.3
-
2
-
-
0038446777
-
Sixteen novel mutations in the Cu/Zn superoxide dismutase gene in amyotrophic lateral sclerosis: A decade of discoveries, defects and disputes
-
Andersen P. M., Sims K. B., Xin W. W. et al. (2003) Sixteen novel mutations in the Cu/Zn superoxide dismutase gene in amyotrophic lateral sclerosis: a decade of discoveries, defects and disputes. Amyotroph. Lateral Scler. Other Motor Neuron Disord. 4, 62-73.
-
(2003)
Amyotroph. Lateral Scler. Other Motor Neuron Disord.
, vol.4
, pp. 62-73
-
-
Andersen, P.M.1
Sims, K.B.2
Xin, W.W.3
-
3
-
-
0027359404
-
Isolation of Hsp90 mutants by screening for decreased steroid receptor function
-
Bohen S. P. and Yamamoto K. R. (1993) Isolation of Hsp90 mutants by screening for decreased steroid receptor function. Proc. Natl Acad. Sci. USA 90, 1I 424-1I 428.
-
(1993)
Proc. Natl. Acad. Sci. USA
, vol.90
-
-
Bohen, S.P.1
Yamamoto, K.R.2
-
4
-
-
0027965073
-
Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity
-
Borchelt D. R., Lee M. K., Slunt H. S. et al. (1994) Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity. Proc. Natl Acad. Sci. USA 91, 8292-8296.
-
(1994)
Proc. Natl. Acad. Sci. USA
, vol.91
, pp. 8292-8296
-
-
Borchelt, D.R.1
Lee, M.K.2
Slunt, H.S.3
-
5
-
-
0344507132
-
Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis
-
Bruening W., Roy J., Giasson B., Figlewicz D. A., Mushynski W. E. and Durham H. D. (1999) Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis. J. Neurochem. 72, 693-699.
-
(1999)
J. Neurochem.
, vol.72
, pp. 693-699
-
-
Bruening, W.1
Roy, J.2
Giasson, B.3
Figlewicz, D.A.4
Mushynski, W.E.5
Durham, H.D.6
-
6
-
-
0031051485
-
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions
-
Bruijn L. I., Becher M. W., Lee M. K. et al. (1997) ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron 18, 327-338.
-
(1997)
Neuron
, vol.18
, pp. 327-338
-
-
Bruijn, L.I.1
Becher, M.W.2
Lee, M.K.3
-
7
-
-
0032544674
-
Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1
-
Bruijn L. I., Houseweart M. K., Kato S., Anderson K. L., Anderson S. D., Ohama E., Reaume A. G., Scott R. W. and Cleveland D. W. (1998) Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science 281, 1851-1854.
-
(1998)
Science
, vol.281
, pp. 1851-1854
-
-
Bruijn, L.I.1
Houseweart, M.K.2
Kato, S.3
Anderson, K.L.4
Anderson, S.D.5
Ohama, E.6
Reaume, A.G.7
Scott, R.W.8
Cleveland, D.W.9
-
8
-
-
0141642203
-
Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice
-
Clement A. M., Nguyen M. D., Roberts E. A. et al. (2003) Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice. Science 302, 113-117.
-
(2003)
Science
, vol.302
, pp. 113-117
-
-
Clement, A.M.1
Nguyen, M.D.2
Roberts, E.A.3
-
9
-
-
0035516124
-
From Charcot to Lou Gehrig: Deciphering selective motor neuron death in ALS
-
Cleveland D. W. and Rothstein J. D. (2001) From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat. Rev. Neurosci. 2, 806-819.
-
(2001)
Nat. Rev. Neurosci.
, vol.2
, pp. 806-819
-
-
Cleveland, D.W.1
Rothstein, J.D.2
-
10
-
-
0035394668
-
Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice
-
Cummings C. J., Sun Y., Opal P., Antalffy B., Mestril R., Orr H. T., Dillmann W. H. and Zoghbi H. Y. (2001) Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice. Hum. Mol. Genet. 10, 1511-1518.
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 1511-1518
-
-
Cummings, C.J.1
Sun, Y.2
Opal, P.3
Antalffy, B.4
Mestril, R.5
Orr, H.T.6
Dillmann, W.H.7
Zoghbi, H.Y.8
-
11
-
-
0030777650
-
Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS
-
Durham H. D., Roy J., Dong L. and Figlewicz D. A. (1997) Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS. J. Neuropathol. Exp. Neurol. 56, 523-530.
-
(1997)
J. Neuropathol. Exp. Neurol.
, vol.56
, pp. 523-530
-
-
Durham, H.D.1
Roy, J.2
Dong, L.3
Figlewicz, D.A.4
-
12
-
-
0028284779
-
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation
-
Gumey M. E., Pu H., Chiu A. Y. et al. (1994) Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science 264, 1772-1775.
-
(1994)
Science
, vol.264
, pp. 1772-1775
-
-
Gumey, M.E.1
Pu, H.2
Chiu, A.Y.3
-
13
-
-
0347928859
-
Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression
-
Hansson O., Nylandsted J., Castilho R. F., Leist M., Jaattela M. and Brundin P. (2003) Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression. Brain Res. 970, 47-57.
-
(2003)
Brain Res.
, vol.970
, pp. 47-57
-
-
Hansson, O.1
Nylandsted, J.2
Castilho, R.F.3
Leist, M.4
Jaattela, M.5
Brundin, P.6
-
14
-
-
0027184721
-
Molecular chaperone functions of heat-shock proteins
-
Hendrick J. P. and Hartl F. U. (1993) Molecular chaperone functions of heat-shock proteins. Annu. Rev. Biochem. 62, 349-384.
-
(1993)
Annu. Rev. Biochem.
, vol.62
, pp. 349-384
-
-
Hendrick, J.P.1
Hartl, F.U.2
-
15
-
-
0037022339
-
Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)
-
Howland D. S., Liu J., She Y. et al. (2002) Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc. Natl Acad. Sci. USA 99, 1604-1609.
-
(2002)
Proc. Natl. Acad. Sci. USA
, vol.99
, pp. 1604-1609
-
-
Howland, D.S.1
Liu, J.2
She, Y.3
-
16
-
-
0034629073
-
Genetic suppression of polyglutamine toxicity in Drosophila
-
Kazemi-Esfarjani P. and Benzer S. (2000) Genetic suppression of polyglutamine toxicity in Drosophila. Science 287, 1837-1840.
-
(2000)
Science
, vol.287
, pp. 1837-1840
-
-
Kazemi-Esfarjani, P.1
Benzer, S.2
-
17
-
-
1942486792
-
Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice
-
Kieran D., Kalmar B., Dick J. R., Riddoch-Contreras J., Burnstock G. and Greensmith L. (2004) Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice. Nat. Med. 10, 402-405.
-
(2004)
Nat. Med.
, vol.10
, pp. 402-405
-
-
Kieran, D.1
Kalmar, B.2
Dick, J.R.3
Riddoch-Contreras, J.4
Burnstock, G.5
Greensmith, L.6
-
18
-
-
3242701496
-
Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria
-
Liu J., Lillo C., Jonsson P. A. et al. (2004) Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron 43, 5-17.
-
(2004)
Neuron
, vol.43
, pp. 5-17
-
-
Liu, J.1
Lillo, C.2
Jonsson, P.A.3
-
19
-
-
0028932259
-
Overexpression of the rat inducible 70-kD heat stress protein in a transgenic mouse increases the resistance of the heart to ischemic injury
-
Marber M. S., Mestril R., Chi S. H., Sayen M. R., Yellon D. M. and Dillmann W. H. (1995) Overexpression of the rat inducible 70-kD heat stress protein in a transgenic mouse increases the resistance of the heart to ischemic injury. J. Clin. Invest. 95, 1446-1456.
-
(1995)
J. Clin. Invest.
, vol.95
, pp. 1446-1456
-
-
Marber, M.S.1
Mestril, R.2
Chi, S.H.3
Sayen, M.R.4
Yellon, D.M.5
Dillmann, W.H.6
-
20
-
-
0037119407
-
Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice
-
Mattiazzi M., D'Aurelio M., Gajewski C. D., Martushova K., Kiaei M., Beal M. F. and Manfredi G. (2002) Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J. Biol. Chem. 277, 29 626-29 633.
-
(2002)
J. Biol. Chem.
, vol.277
-
-
Mattiazzi, M.1
D'Aurelio, M.2
Gajewski, C.D.3
Martushova, K.4
Kiaei, M.5
Beal, M.F.6
Manfredi, G.7
-
21
-
-
0035575761
-
Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: Associated mutations develop motor neuron disease
-
Nagai M., Aoki M., Miyoshi I., Kato M., Pasinelli P., Kasai N., Brown R. H. Jr and Itoyama Y. (2001) Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: associated mutations develop motor neuron disease. J. Neurosci. 21, 9246-9254.
-
(2001)
J. Neurosci.
, vol.21
, pp. 9246-9254
-
-
Nagai, M.1
Aoki, M.2
Miyoshi, I.3
Kato, M.4
Pasinelli, P.5
Kasai, N.6
Brown Jr., R.H.7
Itoyama, Y.8
-
22
-
-
0028815433
-
Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons
-
Pardo C. A., Xu Z., Borchelt D. R., Price D. L., Sisodia S. S. and Cleveland D. W. (1995) Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons. Proc. Natl Acad. Sci. USA 92, 954-958.
-
(1995)
Proc. Natl. Acad. Sci. USA
, vol.92
, pp. 954-958
-
-
Pardo, C.A.1
Xu, Z.2
Borchelt, D.R.3
Price, D.L.4
Sisodia, S.S.5
Cleveland, D.W.6
-
23
-
-
3242703300
-
Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria
-
Pasinelli P., Belford M. E., Lennon N., Bacskai B. J., Hyman B. T., Trotti D. and Brown R. H. Jr (2004) Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria. Neuron 43, 19-30.
-
(2004)
Neuron
, vol.43
, pp. 19-30
-
-
Pasinelli, P.1
Belford, M.E.2
Lennon, N.3
Bacskai, B.J.4
Hyman, B.T.5
Trotti, D.6
Brown Jr., R.H.7
-
24
-
-
17744372861
-
Roles of the heat shock transcription factors in regulation of the heat shock response and beyond
-
Pirkkala L., Nykanen P. and Sistonen L. (2001) Roles of the heat shock transcription factors in regulation of the heat shock response and beyond. FASEB J. 15, 1118-1131.
-
(2001)
FASEB J.
, vol.15
, pp. 1118-1131
-
-
Pirkkala, L.1
Nykanen, P.2
Sistonen, L.3
-
25
-
-
0034089551
-
Mice overexpressing rat heat shock protein 70 are protected against cerebral infarction
-
Rajdev S., Hara K., Kokubo Y., Mestril R., Dillmann W., Weinstein P. R. and Sharp F. R. (2000) Mice overexpressing rat heat shock protein 70 are protected against cerebral infarction. Ann. Neurol. 47, 782-791.
-
(2000)
Ann. Neurol.
, vol.47
, pp. 782-791
-
-
Rajdev, S.1
Hara, K.2
Kokubo, Y.3
Mestril, R.4
Dillmann, W.5
Weinstein, P.R.6
Sharp, F.R.7
-
26
-
-
0028046847
-
Mitochondrial protein import: Biochemical and genetic evidence for interaction of matrix hsp70 and the inner membrane protein MIM44
-
Rassow J., Maarse A. C., Krainer E., Kubrich M., Muller H., Meijer M., Craig E. A. and Pfanner N. (1994) Mitochondrial protein import: biochemical and genetic evidence for interaction of matrix hsp70 and the inner membrane protein MIM44. J. Cell Biol. 127, 1547-1556.
-
(1994)
J. Cell Biol.
, vol.127
, pp. 1547-1556
-
-
Rassow, J.1
Maarse, A.C.2
Krainer, E.3
Kubrich, M.4
Muller, H.5
Meijer, M.6
Craig, E.A.7
Pfanner, N.8
-
27
-
-
15844393658
-
Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
-
Reaume A. G., Elliott J. L., Huffman E. K. et al. (1996) Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury. Nat. Genet. 13, 43-47.
-
(1996)
Nat. Genet.
, vol.13
, pp. 43-47
-
-
Reaume, A.G.1
Elliott, J.L.2
Huffman, E.K.3
-
28
-
-
0027401203
-
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
-
Rosen D. R., Siddique T., Patterson D. et al. (1993) Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362, 59-62.
-
(1993)
Nature
, vol.362
, pp. 59-62
-
-
Rosen, D.R.1
Siddique, T.2
Patterson, D.3
-
29
-
-
0032402093
-
Glutamate potentiates the toxicity of mutant Cu/Zn-superoxide dismutase in motor neurons by postsynaptic calcium-dependent mechanisms
-
Roy J., Minotti S., Dong L., Figlewicz D. A. and Durham H. D. (1998) Glutamate potentiates the toxicity of mutant Cu/Zn-superoxide dismutase in motor neurons by postsynaptic calcium-dependent mechanisms. J. Neurosci. 18, 9673-9684.
-
(1998)
J. Neurosci.
, vol.18
, pp. 9673-9684
-
-
Roy, J.1
Minotti, S.2
Dong, L.3
Figlewicz, D.A.4
Durham, H.D.5
-
30
-
-
0028618296
-
Protein folding and the regulation of signaling pathways
-
Rutherford S. L. and Zuker C. S. (1994) Protein folding and the regulation of signaling pathways. Cell 79, 1129-1132.
-
(1994)
Cell
, vol.79
, pp. 1129-1132
-
-
Rutherford, S.L.1
Zuker, C.S.2
-
31
-
-
0035918258
-
Mutant Cu/Zn-superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis
-
Shinder G. A., Lacourse M. C., Minotti S. and Durham H. D. (2001) Mutant Cu/Zn-superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis. J. Biol. Chem. 276, 12 791-12 796.
-
(2001)
J. Biol. Chem.
, vol.276
-
-
Shinder, G.A.1
Lacourse, M.C.2
Minotti, S.3
Durham, H.D.4
-
32
-
-
0036841876
-
Upregulation of HSP27 in a transgenic model of ALS
-
Vleminckx V., Van Damme P., Coffin K., Delye H., Van Den Bosch L. and Robberecht W. (2002) Upregulation of HSP27 in a transgenic model of ALS. J. Neuropathol. Exp. Neurol. 61, 968-974.
-
(2002)
J. Neuropathol. Exp. Neurol.
, vol.61
, pp. 968-974
-
-
Vleminckx, V.1
Van Damme, P.2
Coffin, K.3
Delye, H.4
Van Den Bosch, L.5
Robberecht, W.6
-
33
-
-
0036076642
-
Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site
-
Wang J., Xu G., Gonzales V., Coonfield M., Fromholt D., Copeland N. G., Jenkins N. A. and Borchelt D. R. (2002) Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site. Neurobiol. Dis. 10, 128-138.
-
(2002)
Neurobiol. Dis.
, vol.10
, pp. 128-138
-
-
Wang, J.1
Xu, G.2
Gonzales, V.3
Coonfield, M.4
Fromholt, D.5
Copeland, N.G.6
Jenkins, N.A.7
Borchelt, D.R.8
-
34
-
-
0032727617
-
Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP 70
-
Warrick J. M., Chan H. Y., Gray-Board G. L., Chai Y., Paulson H. L. and Bonini N. M. (1999) Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP 70. Nat. Genet. 23, 425-428.
-
(1999)
Nat. Genet.
, vol.23
, pp. 425-428
-
-
Warrick, J.M.1
Chan, H.Y.2
Gray-Board, G.L.3
Chai, Y.4
Paulson, H.L.5
Bonini, N.M.6
-
35
-
-
0035664213
-
Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues
-
Watanabe M., Dykes-Hoberg M., Culotta V. C., Price D. L., Wong P. C. and Rothstein J. D. (2001) Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues. Neurobiol. Dis 8, 933-941.
-
(2001)
Neurobiol. Dis
, vol.8
, pp. 933-941
-
-
Watanabe, M.1
Dykes-Hoberg, M.2
Culotta, V.C.3
Price, D.L.4
Wong, P.C.5
Rothstein, J.D.6
-
36
-
-
0029053881
-
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
-
Wong P. C., Pardo C. A., Borchelt D. R., Lee M. K., Copeland N. G., Jenkins N. A., Sisodia S. S., Cleveland D. W. and Price D. L. (1995) An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14, 1105-1116.
-
(1995)
Neuron
, vol.14
, pp. 1105-1116
-
-
Wong, P.C.1
Pardo, C.A.2
Borchelt, D.R.3
Lee, M.K.4
Copeland, N.G.5
Jenkins, N.A.6
Sisodia, S.S.7
Cleveland, D.W.8
Price, D.L.9
-
37
-
-
0037428164
-
Molecular chaperones Hsp90 and Hsp70 deliver preproteins to the mitochondrial import receptor Tom70
-
Young J. C., Hoogenraad N. J. and Hartl F. U. (2003) Molecular chaperones Hsp90 and Hsp70 deliver preproteins to the mitochondrial import receptor Tom70. Cell 112, 41-50.
-
(2003)
Cell
, vol.112
, pp. 41-50
-
-
Young, J.C.1
Hoogenraad, N.J.2
Hartl, F.U.3
|